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迟发性脂质贮积性肌病一例。肌电图、组织化学及超微结构检查结果

A case of late onset lipid storage myopathy. Electromyographic, histochemical and ultrastructural findings.

作者信息

Pinelli P, Poloni M, Nappi G, Scelsi R

出版信息

Eur Neurol. 1975;13(3):273-84. doi: 10.1159/000114683.

DOI:10.1159/000114683
PMID:1149751
Abstract

A 65-year-old woman showed the clinical picture of a myogenic myopathy, confirmed electromyographically. Detailed histopathological, histochemical and ultrastructural studies were performed on deltoid and quadriceps femoris biopsy specimens. Many muscle fibres were found to contain vacuolar spaces with lipid droplets: perivascular inflammatory changes were also observed. The affected fibres were consistently type I. Under the electron microscope the fibres contained large numbers of lipid droplets, usually next to normal mitochondria. A slight improvement occurred after prednisone therapy.

摘要

一名65岁女性表现出肌源性肌病的临床症状,经肌电图检查得以证实。对三角肌和股四头肌活检标本进行了详细的组织病理学、组织化学和超微结构研究。发现许多肌纤维含有带有脂滴的空泡间隙:还观察到血管周围的炎症变化。受影响的纤维始终为I型。在电子显微镜下,这些纤维含有大量脂滴,通常紧邻正常线粒体。泼尼松治疗后病情略有改善。

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